RESUMO

Tuberous sclerosis complex is a group of autosomal disorders characterized by hamartomas and benign neoplastic lesions that invariably affect the central nervous system. We report a case of tuberous sclerosis that is the first presenting ultrastructural findings of this phacomatosis in the Latin American literature. The patient was a 2 year old girl presenting West syndrome non responsive to the clinical treatment with vigabatrin, trileptal and clonazepan, and undergoing left frontal lobectomy. The histopathological and ultrastructural findings were compatible with tuberous sclerosis. These results may help to further understand this controversial phacomatosis, warning to the clinical presentation as West syndrome.

Assuntos

Encéfalo/ultraestrutura , Esclerose Tuberosa/patologia , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Espasmos Infantis/diagnóstico